Lately, the development of medicine has led to the allocation of a group of rare diseases, which have recently been described in the scientific literature and identified as a group of orphan diseases. The purpose of the study was to analyze the possibilities of improving the diagnostics of Churg-Strauss syndrome (CSS) and to improve the effectiveness of treatment according to the existing literature and our own experience of long-term care for patients with eosinophilic granulomatosis with polyangiitis (EGPA) or Churg-Strauss syndrome. EGPA occurs in about 4-10 people in a million population, so each case is of interest. Material and methods. The conduct of 20-year-old male patient was considered during 3 years. The duration of the disease before the diagnosis was 5 years. Anamnesis and medical documents analysis showed a typical CSS debut in the form of allergic rhinitis, nasal polyps and respiratory disorders, which were regarded as bronchitis or bronchial asthma – corresponding to the first phase, which is also called the prodromal or allergic stage of CSS. But CSS could be suspected because of low effectiveness of the therapy with inhaled steroids, lack of effect of antibiotics, and eosinophilia more than 25% that occurs periodically. Results and discussion. The patient was hospitalized in a neurological department of a hospital with a bilateral polyneuritis, neuritis of the fibular nerve with severe pain syndrome. The bilateral infiltrative process was detected in the lungs radiologically and in a smear of peripheral blood – 55 % eosinophilia, ESR – 45 mm per hour, which made it possible, due to the presence of all clinical signs, to diagnose EGPA. Using corticosteroids in a dose of 48 mg metipred led to a rapid reduction of symptoms but a year later, despite receiving maintenance doses of steroids, a patient was hospitalized with an acute exacerbation, and one-sided infiltration in the lungs was detected. Correction of therapy with using corticosteroids and cytostatics allowed to overcome the activity of autoimmune inflammation and to achieve clinical remission. Constant intake of maintenance doses of corticosteroids and cytostatics allowed achieving stabilization of the process in a patient with EGPA. Conclusions. The management of patients with orphan diseases requires special attention from doctors and patient training in taking medication, adherence to the rules of conduct in connection with the peculiarities of a particular pathology.
Keywords: eosinophilic granulomatosis with polyangiitis, diagnosis, treatment
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